Murata K, Morishita S, Nakamuro T, Sugata T, Takayanagi T
Department of Neurology, Nara Medical University.
Rinsho Shinkeigaku. 1991 Feb;31(2):213-5.
We reported a case of hypertrophic neuropathy of adult onset. The pathological change in the sural nerve was decreased axonal population with onion-bulb formation. On examination, there were enlarged nerve on palpation and she was found to have distal muscle atrophy, weakness and sensory loss. The deep tendon reflexes of extremities were weak. The first clinical feature of this patient was mainly polyneuropathy. The lower limbs were slightly spastic and plantar responses were extensor bilaterally. There was also sensory level at C6 level. After 1.5 years from first examination, she had shown myelopathy. Magnetic resonance imaging (MRI) of the spine showed marked thickening of the nerve roots and it revealed the compression of the spinal cord by enlarged nerve roots from C2 to C6 level. The compression syndrome of the patients with hypertrophic neuropathy was unclear at the onset in this case. MRI study of the spinal cord would be very beneficial to disclose subclinical myelopathy associated with hypertrophic neuropathy, as indicated in this report.
我们报告了一例成人起病的肥厚性神经病病例。腓肠神经的病理改变为轴突数量减少并伴有洋葱球样形成。检查时，触诊发现神经增粗，且发现她有远端肌肉萎缩、无力和感觉丧失。四肢的深腱反射减弱。该患者的首要临床特征主要为多发性神经病。双下肢轻度痉挛，双侧跖反射为伸性。在C6水平也存在感觉平面。首次检查1.5年后，她出现了脊髓病。脊柱磁共振成像（MRI）显示神经根明显增厚，并显示从C2至C6水平的增大神经根对脊髓造成压迫。在该病例中，肥厚性神经病患者的压迫综合征在起病时并不明确。正如本报告所示，脊髓MRI检查对于发现与肥厚性神经病相关的亚临床脊髓病非常有益。
